Phacoemulsification, pinhole pupilloplasty, and pre-Descemet抯 endothelial keratoplasty for keratoconus with Fuchs� endothelial dystrophy

The comorbidity of keratoconus with Fuchs� endothelial dystrophy with cataract is a rare clinical combination. We present an amalgamation of surgical techniques to manage the above clinical conditions and its complications in single setting. The modified triple procedure, namely, the phacoemulsification, pinhole pupilloplasty, and pre-Descemet抯 endothelial keratoplasty (PDEK) in the order of description is followed in single sitting. Lens removal by phacoemulsification, correction of irregular astigmatism by pinhole pupilloplasty (pinhole optics), and exchanging the endothelial layer for PDEK forms the main segments of the triple procedure. This combination of techniques may decrease the risk of multiple surgeries and its related complications. Moreover, it will allow the patient for faster visual rehabilitation by improving the uncorrected visual acuity and visual quality.

Clinical profile and demographic distribution of Fuchs’ endothelial dystrophy: An electronic medical record–driven big data analytics from an eye care network in India

Purpose: To describe the demographics and clinical profile of Fuchs’ endothelial corneal dystrophy (FECD) in patients presenting to a multi?tiered ophthalmology hospital network in India. Methods: This cross?sectional hospital?based study included 3,082,727 new patients presenting between August 2010 and December 2021. Patients with a clinical diagnosis of FECD in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 2570 (0.08%) patients were diagnosed with FECD. The majority of the patients were female (65.53%) and were predominantly adults (99.92%). The most common age group at presentation was during the seventh decade of life with 867 patients (33.74%). The overall prevalence was higher in patients from a higher socioeconomic status (0.1%) presenting from the urban geography (0.09%) and in retired individuals (0.4%). About half of the 5,140 eyes had mild or no visual impairment (< 20/70) in 2643 eyes (51.42%) followed by moderate visual impairment (>20/70 to 20/200) in 708 eyes (13.77%). The average logMAR was 0.61 ± 0.81 at presentation. The most documented corneal signs were guttae (76.63%), corneal scar (23%) and stromal edema (21.73%). The most associated ocular comorbidity was cataract (47.32%) followed by glaucoma (5.39%). More than a tenth of the affected eyes required a surgical intervention of endothelial keratoplasty (15.58%). Conclusion: FECD more commonly affects females presenting during the seventh decade of life. Majority of the eyes had mild or no visual impairment and endothelial keratoplasty is warranted in a tenth of the affected eyes

Comportamiento de los pacientes con distrofia corneal endotelial de Fuchs en la provincia Camagüey

Introducción: La distrofia corneal endotelial de Fuchs se trata de un trastorno degenerativo específico, bilateral y progresivo del endotelio corneal, es la más frecuente pero no siempre es diagnosticada en sus etapas iniciales en las consultas de oftalmología general. Objetivo: Describir el comportamiento clínico de pacientes con distrofia corneal endotelial de Fuchs en la provincia Camagüey. Métodos: Se realizó un estudio observacional, descriptivo, transversal en el Centro Oftalmológico del Hospital Universitario Manuel Ascunce Domenech en la provincia Camagüey desde noviembre 2019 hasta junio 2021. El universo de estudio estuvo constituido por todos los pacientes que asistieron durante el periodo de estudio y la muestra la conformaron 19 pacientes (38 ojos) quienes cumplieron con los criterios de inclusión y exclusión. Las variables estudiadas fueron edad, sexo, color de la piel, agudeza visual con corrección, asociación con glaucoma, paquimetría, biomicroscopía del segmento anterior, microscopía endotelial, microscopía confocal, estadio de la enfermedad y tipo de tratamiento aplicado. Resultados: Predominaron los pacientes entre 40 y 59 años de edad, el sexo femenino y color blanco de la piel. Sobresalió la visión útil, los valores de paquimetría altos y asociados al glaucoma. Se constató la presencia de guttas, edema corneal, bajo conteo celular con polimorfismo y polimegatismo. El estadio 2 estuvo en 47,4 % y el tratamiento médico se aplicó en el 97,4 %. Conclusiones: La distrofia aparece con más frecuencia después de los 40 años de edad, en sexo femenino y color blanco de piel. Predominó la visión útil, valores altos de paquimetrías y asociación con glaucoma. En la biomicroscopía del segmento anterior predominaron las guttas y el edema estromal y la microscopía endotelial y confocal se caracterizaron en su mayoría por el bajo conteo celular, las guttas, polimorfismo y polimegatismo. Prevaleció el estadio 2 y el tratamiento médico.

Introduction: Fuchs endothelial corneal dystrophy is a specific, bilateral and progressive degenerative disorder of the corneal endothelium, it is the most frequent but it is not always diagnosed in its initial stages in general ophthalmology consultations. Objective: To describe the clinical behavior of patients with Fuchs endothelial corneal dystrophy in Camagüey province. Methods: A cross-sectional descriptive observational study was carried out at the Ophthalmological Center of the Manuel Ascunce Domenech University Hospital in Camagüey in the period from November 2019 to June 2021. The study universe consisted of all the patients who attended during the study period and the sample was made up of 19 patients (38 eyes) who met the inclusion and exclusion criteria. The variables studied were age, sex, skin color, corrected visual acuity, association with glaucoma, pachymetry, anterior segment biomicroscopy, endothelial microscopy, confocal microscopy, disease stage, and type of treatment applied. Results: Patients between 40 and 59 years of age, female sex, and white skin color predominated. Useful vision stood out, high pachymetry values and associated with glaucoma, the presence of guttas, corneal edema, low cell count with polymorphism, and polymegatism was confirmed. Stage 2 was 47.4% and medical treatment was applied in 97.4%. Conclusions: Dystrophy appears more frequently after 40 years of age, in females and white skin persons. Useful vision, high pachymetry values, and association with glaucoma prevailed. In the biomicroscopy of the anterior segment, guttas and stromal edema predominated, and endothelial and confocal microscopy were mostly characterized by low cell count, guttas, polymorphism, and polymegatism. Stage 2 and medical treatment prevailed.

Aplanamento corneano após DMEK associado a facoemulsificação em paciente com distrofia endotelial de Fuchs, ceratocone e catarata / Corneal flattening after simultaneous DMEK and phacoemulsification in patient with Fuchs endothelial dystrophy, keratoconus and cataract combined

Resumo Descrição de relato de caso de paciente com ceratocone (KC), Distrofia Endotelial de Fuchs (DEF) e catarata concomitantes com descompensação corneana submetido a Ceratoplastia Lamelar Posterior pela técnica Descemet's Membrane Endothelial Keratoplasty (DMEK) associado a facoemulsificação com implante de lente intraocular (LIO). Observou-se aplanamento corneano significativo no pós-operatório e acuidade visual final sem correção de 20/25 . Destaca-se a possibilidade do DMEK como alternativa à Ceratoplastia Penetrante (Penetrating Keratoplasty - PK) em casos de DEF e KC associados. Aplanamento corneano pode ocorrer neste grupo de pacientes, o que pode resultar em imprevisibilidade refracional no cálculo do poder da LIO ao se optar por facectomia combinada. Neste relato, apesar da modificação da curvatura corneana após cirurgia combinada de DMEK com facectomia, a acuidade visual final sem correção foi satisfatória, demonstrando a possibilidade de sucesso desta abordagem na coexistência de DEF, KC e Catarata. Entretanto, a possibilidade de mudança significativa na curvatura corneana deve ser considerada em pacientes com KC, edema de córnea secundário a DEF e catarata, na decisão de cirurgia simultânea ou em dois tempos.

Abstract This is a case report of a patient with concomitant Keratoconus (KC), Fuchs Endothelial Dystrophy (FED) and cataract with corneal decompensation submitted to Posterior Lamellar Corneal surgery (Descemet's Membrane Endothelial Keratoplasty - DMEK) associated with phacoemulsification with intraocular lens (IOL) implantation. Corneal flattening and uncorrected visual acuity of 20/25 was observed in the postoperative period. We reasure the viability of DMEK as an alternative to Penetrating Keratoplasty (PK) in cases of associated DEF and KC associated. Changes in corneal curvature may occur in this group of patients and lead to the possibility of refractive unpredictability in IOL calculation when performing a combined cataract surgery. Despite unexpected corneal flattening, satisfactory final visual acuity was achieved, demonstrating the possibility of success of this approach in the coexistence of the three conditions. Nonetheless, the possibility of corneal curvature changes should be considered in patients with KC and corneal decompensation due to FED in decision making, regarding simultaneous or sequential surgical approach.

Descemet membrane endothelial keratoplasty in multifocal pseudophakic eyes / Ceratoplastia endotelial da membrana de Descemet em olhos pseudofácicos multifocais

ABSTRACT Purpose: This report describes the use of Descemet membrane endothelial keratoplasty for the management of endothelial decompensation after multifocal intraocular lens implantation. Methods: In this retrospective study, we reviewed and assessed the surgical outcomes of 9 patients (9 eyes) who underwent Descemet membrane endothelial keratoplasty after multifocal intraocular lens implantation. Results: Corneal edema occurred due to Fuchs endothelial corneal dystrophy (n=3), pseudophakic bullous keratopathy (n=3), Descemet's membrane detachment (n=2), and toxic anterior segment syndrome (n=1). The Descemet membrane endothelial keratoplasty surgeries were uneventful in all eyes, but rebubbling procedures were necessary in 2 eyes. One month after the surgery, all the corneas were clear. After 6 months, excluding 1 eye with amblyopia, the mean distance corrected visual acuity was 0.10 logMAR, with all eyes achieving 0.18 logMAR or better. Conclusions: This is the first report of Descemet membrane endothelial keratoplasty after multifocal intraocular lens implantation, and it suggests that good results can be achieved without multifocal intraocular lens exchange.

RESUMO Objetivo: Descrever o uso da ceratoplastia endotelial da membrana de Descemet para manejar descompensação endotelial após implante de lente intraocular multifocal. Métodos: Neste estudo retrospectivo, foram revisados e avaliados os resultados cirúrgicos de 9 olhos de 9 pacientes que foram submetidos a ceratoplastia endotelial da membrana de Descemet para manejar descompensação endotelial após implante de lente intraocular multifocal. Resultados: Descompensação endotelial ocorreu por distrofia endotelial de Fuchs (n=3), ceratopatia bolhosa do pseudofácico (n=3), descolamento da membrana de Descemet (n=2) e síndrome tóxica do segmento anterior (n=1). No ato per operatório de todos os olhos não houve intercorrência, com injeção de ar sendo necessário em dois olhos no pós-operatório por descolamento parcial do enxerto. Um mês após a cirurgia, todas as córneas estavam claras. Após seis meses, excluindo um olho com ambliopia, a acuidade visual média corrigida para longe foi de 0,10 logMAR, com todos os olhos atingindo 0,18 logMAR ou melhor. Conclusões: Este é o primeiro relato de ceratoplastia endotelial da membrana de Descemet após implante de lente intraocular multifocal, sugerindo que bons resultados podem ser alcançados sem a troca da lente intraocular multifocal.

Relevância da biomecânica da córnea no glaucoma / Relevancy of corneal biomechanical in glaucoma

Relato de caso de uma paciente, com córnea espessa, por distrofia de Fuchs em fase inicial, ainda com transparência corneana preservada (edema corneano subclínico), associado à pressão intraocular (PIO) normal por tonometria de aplanação de Goldman (TAG), que teve entretanto, o diagnóstico de glaucoma primário de ângulo aberto com base em alterações estruturais do nervo óptico. O estudo tomográfico demonstrou padrão de paquimetria espacial compatível com edema subclínico. O estudo biomecânico da córnea com o ORA (Ocular Response Analyzer, ® Reichert), associado à medida corrigida da PIO, possibilitou o entendimento da influência da córnea, que apesar de mais espessa, levava a uma TAG falsamente reduzida (hipoestimada). O estudo da PIO com sistema de tonografia digital de contorno (PASCAL) corroborou com os achados do ORA. Este exemplo ressalta a importância de novas tecnologias na avaliação de pacientes com suspeita de glaucoma, e destaca que a correção da TAG, com base em algoritmos lineares relacionados com a paquimetria central apenas, pode determinar sérios erros de interpretação clínica.

Case report of a patient, with a thick cornea, for Fuchs dystrophy in its early stages, yet with preserved corneal transparency (subclinical corneal edema), associated with normal intraocular pressure (IOP) by Goldman applanation tonometry (GAT), which was however, the diagnosis of primary open-angle glaucoma on the basis of structural changes of the optic nerve. The tomographic study showed a pattern of spatial pachymetry compatible with subclinical edema. The corneal biomechanical study with ORA (Ocular Response Analyzer, Reichert ®) associated with corrected IOP measurement, allowed the understanding of the corneal influence, which, although thicker, leading to a falsely low TAG (underestimated). The IOP study with tonography system digital (PASCAL) corroborated with the findings of ORA. This example highlights the importance of new technologies in patients' evaluation with suspected glaucoma, and highlights that the correction of TAG, based on linear algorithms related to central corneal thickness alone, can determine serious errors in clinical interpretation.

Relevância da biomecânica da córnea no glaucoma / Relevancy of corneal biomechanical in glaucoma

Relato de caso de uma paciente, com córnea espessa, por distrofia de Fuchs em fase inicial, ainda com transparência corneana preservada (edema corneano subclínico), associado à pressão intraocular (PIO) normal por tonometria de aplanação de Goldman (TAG), que teve entretanto, o diagnóstico de glaucoma primário de ângulo aberto com base em alterações estruturais do nervo óptico. O estudo tomográfico demonstrou padrão de paquimetria espacial compatível com edema sub-clínico. O estudo biomecânico da córnea com o ORA (Ocular Response Analyzer, ® Reichert), associado à medida corrigida da PIO, possibilitou o entendimento da influência da córnea, que apesar de mais espessa, levava a uma TAG falsamente reduzida (hipoestimada). O estudo da PIO com sistema de tonografia digital de contorno (PASCAL) corroborou com os achados do ORA. Este exemplo ressalta a importância de novas tecnologias na avaliação de pacientes com suspeita de glaucoma, e destaca que a correção da TAG, com base em algoritmos lineares relacionados com a paquimetria central apenas, pode determinar sérios erros de interpretação clínica.

Case report of a patient, with a thick cornea, for Fuchs dystrophy in its early stages, yet with preserved corneal transparency (subclinical corneal edema), associated with normal intraocular pressure (IOP) by Goldman applanation tonometry (GAT), which was however, the diagnosis of primary open-angle glaucoma on the basis of structural changes of the optic nerve. The tomographic study showed a pattern of spatial pachymetry compatible with subclinical edema. The corneal biomechanical study with ORA (Ocular Response Analyzer, Reichert ®) associated with corrected IOP measurement, allowed the understanding of the corneal influence, which, although thicker, leading to a falsely low TAG (underestimated). The IOP study with tonography system digital (PASCAL) corroborated with the findings of ORA. This example highlights the importance of new technologies in patients' evaluation with suspected glaucoma, and highlights that the correction of TAG, based on linear algorithms related to central corneal thickness alone, can determine serious errors in clinical interpretation.

Axenfeld-Rieger anomaly and corneal endothelial dystrophy: a case series / Anomalia de Axenfeld-Rieger e distrofia corneana endotelial: uma série de casos

Report of a study of a family with a remarkable combination of endothelial corneal dystrophy, and anterior chamber dysgenesis classified as Axenfeld-Rieger anomaly. A 56 year-old female had blurred vision complaints. A slit lamp evaluation showed a bilateral posterior embryotoxon and guttata with corneal edema, Descemet membrane's folds, characterizing Fuchs endothelial dystrophy. A 27 year-old daughter was asymptomatic. The biomicroscopic exam disclosed a bilateral nasal and temporal posterior embryotoxon and a central guttata. Gonioscopy showed iridocorneal strands with a prominent Schwalbe´s line. The intraocular pressure was normal. Her sister, a 25 year-old female presented very similar abnormalities along with her brother (22 years old). A 19 year-old female sister presented the iridocorneal angle alterations, without Fuchs endothelial dystrophy, until this moment. This paper presents a family with a central cornea guttata or Fuchs dystrophy associated with Axenfeld-Rieger anomaly. Two different inherited diseases appearing together in an entire family may suggest a single molecular and genetic etiology, with high penetrance.

Relato dos resultados do estudo de uma família com distrofia corneana endotelial associada a uma disgenesia do segmento anterior, a anomalia de Axenfeld-Rieger. Paciente de 56 anos, feminina, queixava-se de visão borrada. O exame na lâmpada de fenda evidenciou embriotoxo posterior bilateral e guttata no centro da córnea, edema estromal e dobras na membrana de Descemet, caracterizando distrofia endothelial de Fuchs. A filha de 27 anos estava assintomática. A biomicroscopia revelou embriotoxo posterior nasal e temporal bilateral e guttata central. A gonioscopia evidenciou processos iridocorneanos até a linha de Schwalbe, que se encontrava anteriorizada e proeminente. A pressão intra-ocular estava normal. Não foram observadas alterações sistêmicas. Sua irmã, 25 anos, sexo feminino, apresentava alterações muito similares, assim como seu irmão (22 anos). Outra irmã de 19 anos apresentava alterações do ângulo iridocorneano, porém sem distrofia de Fuchs, até o presente. Este trabalho relata os resultados de estudo de uma família com córnea guttata central ou distrofia de Fuchs associada à anomalia de Axenfeld-Rieger. Duas doenças hereditárias com expressão simultânea em toda a família sugerem uma etiologia molecular e genética única, com alta penetrância.